Key Points
Figure 1. Algorithm for Respiratory Management
Diagnosis: ALS
a
Symptoms suggestive of nocturnal hypoventilation: frequent arousals, morning headaches, excessive daytime
sleepiness, vivid dreams
b
If noninvasive ventilation is not tolerated or accepted in the setting of advancing respiratory compromise, consider
invasive ventilation or referral to hospice.
Noninvasive ventilation
tolerated?
Orthopnea or SNP < 40 cm or
MIP < -60 cm or abnormal
nocturnal oximetry or FVC < 50%
Consider noninvasive ventilation
Further education regarding
documented benefits.
Evaluate reasons for
noncompliance
Discuss invasive
ventilation
Suction machine
Manual assisted cough
Mechanical inexsufflator
Treat sialorrhea/phlegm
NO YES
Ongoing evaluations and
adjustment of pressures
Unable to maintain
pO
2
> 90%, pCO
2
< 50 mmHg
or unable to manage secretions
Successful
Hospice referral
for palliative care
Reintroduce noninvasive
ventilation
Î Amyotrophic lateral sclerosis (ALS) is a relentlessly progressive, paralyzing,
neurodegenerative disease. It is characterized by loss of motor neurons in the
spinal cord, brainstem, and motor cortex.
Î Most patients with ALS die within 2 to 5 years of onset.
ÎThe mainstay of ALS management is symptomatic treatment and palliative care.
Î Riluzole has FDA approval for slowing disease progress in ALS.
Î Multidisciplinary clinics are the optimal means to coordinate ALS treatment.
PCEF < 270 L/min
Symptom evaluation
a
and PFTs
Initiate noninvasive ventilation orientation
Pneumovax and flu vaccine
Text in bold = evidence-based
Text in italics = consensus-based
FVC, forced vital capacity;
SNP, sniff nasal pressure;
MIP, maximal inspiratory
pressure; PCCEF, peak
cough expiratory flow
Not Successful
b
